tbuckley/cabot-search huggingface dataset:
Abstract 12736: Cardiac Fludeoxyglucose-18 Positron Emission Tomography in Genotype-Positive Arrhythmogenic Cardiomyopathy
Authors: Andrew S Tseng, Ramin Garmany, Ciorsti MacIntyre, Christopher J McLeod, Leslie T Cooper, Michael J Ackerman, Konstantinos Siontis, John Giudicessi
Journal: Circulation (2022)

Introduction: Myocardial inflammation likely contributes to the pathogenesis of arrhythmogenic cardiomyopathy (ACM), a clinically and genetically heterogenous disorder characterized by ventricular dysfunction and potentially life-threatening ventricular arrhythmias. Due to phenotypic overlap, some patients with genetic ACM may have been evaluated for an underlying inflammatory cardiomyopathy, namely cardiac sarcoidosis. However, the cardiac fludeoxyglucose (FDG) positron emission tomography (PET) findings in ACM patients have not been assessed systematically. Methods: All genotype-positive patients in the Mayo Clinic ACM registry who received a cardiac FDG PET were included in this study. The cardiac FDG PET scan consisted of a resting myocardial perfusion scan with N13-ammonia as well as a F18-FDG scan after a high-fat diet to suppress physiologic FDG uptake. Pertinent demographic, genetic, imaging, and outcome data were extracted from the medical record. Results: Collectively, 12 (75% female) genotype-positive ACM patients received a cardiac PET FDG scan as part of their clinical evaluation (median age at scan 45.3 years [IQR 38.9-62.1]). Amongst these patients, pathogenic/likely pathogenic variants were detected in LMNA (n=7), DSP (n=3), FLNC (n=1), and PLN (n=1). Of note, 6/12 (50%) had abnormal myocardial FDG uptake, including diffuse (entire myocardium) uptake in 2/6 (33%), focal (1-2 segments) uptake in 2/6 (33%) and patchy (3+ segments) in 2/6 (33%). Median myocardial standardized uptake value ratio was 2.1 (IQR 1.5-2.6). Interestingly, LMNA -positive patients accounted for 3 out of 6 (50%) positive studies (diffuse uptake in 2 and focal uptake in 1). Conclusion: Myocardial FDG uptake is common in genetic ACM patients undergoing cardiac FDG PET. This finding further supports the role of myocardial inflammation in genetic ACM and demonstrates the ability of cardiac FDG PET to readily detect this phenomenon. Further investigation is needed to determine (i) the role of cardiac FDG PET in the evaluation of genetic ACM, (ii) if FDG uptake in genetic ACM has prognostic value and (iii) whether the attenuation of myocardial inflammation with immunosuppressive agents can impact the clinical course of inflammatory genetic ACMs.
Abstract 10954: Average Annual Out-of-Pocket Cost of Guideline Directed Medical Therapy for Heart Failure
Authors: Hassan A Alhassan, Harriet Akunor, Aiyer Aryan, Bruce L Rollman, Jing Luo, Gavin W Hickey
Journal: Circulation (2022)

Introduction: The 2022 multi-society heart failure guidelines underscore the importance of initiating and maintaining patients on Guideline Directed Medical Therapy (GDMT). Cost transparency plays a key role in the shared-decision making process, however, national estimates of out-of-pocket (OOP) cost for GDMT for Heart Failure (HF) are lacking Methods: Using data from the Medical Expenditure Panel Survey (MEPS) 2019, we estimated the average annual OOP cost for each GDMT drug class: cardio-selective Beta-Blockers (BB), Angiotensin-converting enzyme inhibitors (ACEi)/Angiotensin II receptor blockers (ARB)/Angiotensin Receptor-Neprilysin Inhibitor (ARNi), Mineralocorticoid Receptor Antagonists (MRA), and Sodium-glucose cotransporter-2 inhibitors (SGLT2i). We also estimated average annual OOP costs for recommended GDMT combinations: Quadruple therapy, quadruple with ARNI alone, and various combinations of triple therapy. Cost estimates were also stratified by insurance status. Results: Of 28,512 MEPS participants, we found 1,954 with a prescription for a BB, 3,642 with at least 1 script for an ACEi/ARB, 42 participants on ARNI, 280 with at least 1 script for an MRA, and 140 with at least 1 script for an SGLT2i. Average annual OOP costs for each GDMT drug class was: $41 (95%CI $34-$48) for BB, $29 (95%CI $26-$32) for ACEi/ARB, ARNi alone estimated at $192 (95%CI $111-$274), $32 (95%CI $25-$39) for MRA, and $151 (95%CI $110-$191) for SGLT2i. The average annual OOP cost for quadruple therapy in 2019 was $253, $292 for privately insured, $159 for those on public insurance, and $975 for the uninsured. Quadruple therapy with ARNI alone on average added ~$200 to the annual OOP cost. For triple therapies, a combination of MRA, BB, and ACEi/ARB was $282 less compared to a combination of BB, ARNI, and SGLT2i. Conclusions: The average annual OOP for GDMT was substantially mitigated by insurance coverage. Including estimates of GDMT cost and the protective role of health insurance coverage in the shared decision-making process might increase the proportion of heart failure patients initiated and maintained on GDMT.
Abstract 10950: Prognostic Utility of Left Atrio-Ventricular Deformation Analysis in Heart Failure Patients Underwent Cardiac Resynchronization Therapy
Authors: Masataka Sugahara, Kyung-Duk Min, Takanao Mine, Masanori Asakura, Masaharu Ishihara
Journal: Circulation (2022)

Introduction: Left atrial (LA) adverse remodeling is a prognostic marker in patients with heart failure. Although left ventricular (LV) deformation analysis by speckle tracking method has been shown as a useful predictive marker after cardiac resynchronization therapy (CRT), the prognostic impact of baseline left atrio-ventricular functional deterioration on the outcome of patients underwent CRT implantation. Hypothesis: We hypothesized that a combination of LV and LA deformation assessments by speckle-tracking strain is of additive prognostic value in patients with CRT. Methods: We retrospectively analyzed 71 participants who received CRT implantation in the clinical guidelines. Global longitudinal strain (GLS) was defined as averaged strain from 3 apical views. LA strain (LAS) was calculated as the average strain using apical two- and four-chamber views. The endpoint was composite outcome of death, hospitalization due to worsening HF for 5 years after CRT implantation. Results: Out of 71 patients aged 68 ± 11 years with 162±24ms of QRS duration and 30±8% of LV ejection fraction, 40 patients (56%) met the endpoint. The patients were stratified using median value of LAS (-15%) and GLS (-8%). LAS and GCS remained significantly associated with poor outcome after adjusting for LVEF (LAS hazard ratio, 0.92; p&lt;0.001; GLS hazard ratio, 1.15; p = 0.01). Kaplan-Meier analysis showed that the subgroup of patients with both lower median values of LAS and GLS had the worst outcomes over 5 years (Figure, Log-rank, p = 0.008). In the sequential Cox model, predictive value of baseline LV ejection fraction (χ2 = 4.9) was improved by the addition of GLS (χ2 = 9.2, p = 0.037), and further improved by the addition of LAS (χ2 = 15.8, p = 0.005). C-statistics also improved from 0.62 to 0.73. Conclusions: Baseline left atrio-ventricular functional deterioration by deformation analysis were significantly associated with poor outcome after CRT implantation.
Abstract 12675: A Modified Classification Scheme for Chaga's Cardiomyopathy Incorporating Cardiac Magnetic Resonance Imaging
Authors: Alexandra Smith, Emilio Fentanes, Rosco Gore, Kelvin Bush
Journal: Circulation (2022)

Introduction: The public health concerns from Chaga’s disease warrant improved cardiovascular imaging efforts. We present a novel and modified Chaga’s staging classification incorporating cardiac magnetic resonance imaging (CMR) to aid in diagnosing cardiomyopathies (CMs). (Figure 1) Case Presentation: An 18 year old asymptomatic military basic trainee screened serology positive for Trypanosoma Cruzi. A native of South Texas, he had frequent exposure to many triatome bugs without any history of chagoma or bites. Electrocardiogram showed sinus bradycardia, first degree atrioventricular delay, right bundle branch block with a left anterior fascicular block. Transthoracic echocardiogram (TTE) demonstrated a preserved left ventricular ejection fraction (LVEF), no wall motion abnormalities or valvular disease. Cardiopulmonary exercise testing demonstrated the trainee prematurely reaching anaerobic threshold. CMR was performed to evaluate specifically for delayed gadolinium enhancement and chamber size derangements for prognostication and military disposition purposes. CMR demonstrated mild left ventricular dilation, LVEF 65%, no late gadolinium enhancement, normal T1 relaxation time of 932 milliseconds, and normal T2 weighted images. The exposure history, positive serologies, electrocardiogram, and early ventricular remodeling were consistent with a Chaga’s dilated CM revealed with CMR improved spatial resolution. Case Outcome: Military standards currently prohibit cardiomyopathies from entering active duty service. The trainee was discharged and referred for medical therapy. Conclusions: We propose that CMR be incorporated into the Chaga’s classification schemes to (1) identify structural disease not apparent by TTE, (2) recognize fibrotic or inflammatory myocardial involvement which is prognosis altering, and (3) diagnose early involvement of CM which could benefit from earlier anti-parasitic therapy.
Abstract 12690: Mavacamten Treatment in Patients With Obstructive and Nonobstructive Hypertrophic Cardiomyopathy: A Pooled Safety Analysis of 5 Clinical Trials
Authors: David Fermin, Sara Saberi, Theodore P Abraham, Iacopo Olivotto, Ahmad Masri, Amy J Sehnert, Neil Minton, Shawna Fox, Richard G Bach
Journal: Circulation (2022)

Introduction: Mavacamten, a cardiac myosin inhibitor, is approved by the US FDA for the treatment of adults with symptomatic obstructive HCM. We present results from an integrated analysis of pooled mavacamten safety data from clinical studies in patients (pts) with obstructive and nonobstructive HCM. Methods: Pts who received ≥1 dose of mavacamten in phase 2 and 3 studies (PIONEER-HCM [end of study date: Jan 12, 2018]; MAVERICK-HCM [Jan 7, 2020]; EXPLORER-HCM [Jul 1, 2020]) and ongoing extension studies (PIONEER-OLE and MAVA-LTE [data cut-off date: Aug 31, 2021]) comprised the pooled analysis population. Results: Data were pooled from a total of 314 unique pts with obstructive (n=260) or nonobstructive (n=54) HCM. Baseline characteristics are shown in the Table. Cumulative time on study (parent + extension) was ≥6 mo for 91% of pts. The mean cumulative duration of mavacamten exposure was 20.2 mo (range: 0.3, 42.5; median: 20.7 mo); 5.4% of pts permanently discontinued treatment due to treatment-emergent adverse events (TEAEs). Total cumulative exposure was 527.6 pt-yrs, and the median average daily dose of mavacamten was 5.7 mg. Generally similar proportions of pts on mavacamten vs placebo experienced grade ≥3, serious, or drug-related TEAEs; few pts experienced TEAEs that led to study discontinuation or drug interruption ( Table ). The exposure-adjusted incidence of adjudicated events in EXPLORER-HCM and MAVA-LTE of heart failure and new-onset atrial fibrillation were 1.42 and 3.07 per 100 pt-yrs, respectively. As previously reported, 4 deaths occurred across studies: 3 in the mavacamten arm due to TEAEs (bacterial endocarditis, cardiac arrest, and acute myocardial infarction [all, n=1]), with none considered related to study drug, and 1 in the placebo arm (sudden death). Conclusion: Integrated evaluation of existing trial data in HCM show that mavacamten is safe and generally well tolerated, irrespective of the presence of obstruction and across multiple doses.
Abstract 11846: Factors Influencing Survival in Adult Patients With Congenital Heart Disease Considered for Transplant: A Single Center Study
Authors: Charith Ratnayake, Gavin W Hickey, Morgan Hindes, Anita Saraf
Journal: Circulation (2022)

Introduction: Adult congenital heart disease (ACHD) patients pose unique challenges in identifying time for transplantation and factors influencing outcomes. Hypothesis: Survival outcomes in ACHD patients depend on hemodynamic, functional, and laboratory parameters. Methods: We performed a retrospective chart review of adult patients (&gt;18y) with CHD evaluated for heart or heart+ transplant between November 2004 and July 2014 and evaluated outcomes until December 2020 at University of Pittsburgh Medical Center. Multiple clinical parameters were evaluated to identify factors influencing outcomes for up to 10 years after consideration for transplant. Kruskal-Wallis nonparametric analysis was conducted to compare differences between variables. Results: 58 adult patients with congenital heart disease (CHD), age range 20-55 years were evaluated. D-TGA with atrial switch operation (20.7%), tetralogy of Fallot/pulmonary atresia (15.5%) and tricuspid atresia (13.8%) were the most common diagnosis for transplant. Of the 58 patients, 5 (8.6%) were deferred, 42 (72.4%) were listed for transplant and 11 (18.9%) were declined for transplant. Statistically significant factors affecting listing decision are included in table 1. Declined patients showed the highest 1-year mortality (18.2%), whereas waitlisted patients showed the highest 10-year mortality (84.2%). In the post-transplantation period, 30 day survival was 87.0%, 1-yr was 82.6%, and 5-yr and 10 year was 69.6%. 1-year post-transplant survival was influenced by pre-transplant end diastolic pressures, use of advanced life support, and right atrial pressure. There was no significant difference in post-transplant survival between single ventricle versus bi-ventricular patients. Conclusions: We have identified clinical factors that influence survival outcomes in ACHD patients considered for transplantation, These can applied while making transplant decisions and improve outcomes
Abstract 10446: Prognostic Significance of Left Ventricular Ejection Fraction in Atrial Fibrillation Patients Without Pre-Existing Heart Failure
Authors: Yasuhiro Hamatani, Moritake Iguchi, Kimihito Minami, Kenjiro Ishigami, Syuhei Ikeda, KOSUKE DOI, Akiko Fujino, Nobutoyo Masunaga, Masahiro Esato, Hikari Tsuji, Hiromichi Wada, Koji Hasegawa, Hisashi Ogawa, Mitsuru Abe, Masaharu Akao
Journal: Circulation (2022)

Introduction: Atrial fibrillation (AF) increases the risk of heart failure (HF). However, little focus has been placed on the prevention of HF in patients with AF. Hypothesis: Left ventricular ejection fraction (LVEF) is an established echocardiographic parameters in patients with HF. We hypothesized that LVEF is a useful predictor for incident HF in AF patients even without HF. Methods: The Fushimi AF Registry is a community-based prospective survey of AF patients in Fushimi-ku, Kyoto, Japan. After excluding patients without pre-existing HF (defined as having one of the following; prior HF hospitalization, New York Heart Association class ≥2, or LVEF &lt;40%), we investigated 2,459 patients with the data of LVEF at enrollment. We divided the patients into 3 groups stratified by LVEF (mildly reduced LVEF [40-49%], below normal LVEF [50-59%], and normal LVEF [≥60%]), and compared the backgrounds and incidence of HF hospitalization between the groups. Results: Of 2,459 patients (mean age: 72.4±10.5 years, female: 917 [37%], paroxysmal AF: 1,405 [57%], and mean CHA 2 DS 2 -VASc score: 3.0±1.6), the mean LVEF was 66±8% (mildly reduced LVEF: 114 [5%], below normal LVEF: 300 [12%] and normal LVEF: 2,045 [83%], respectively). Patients with lower LVEF demonstrated lower prevalence of female and paroxysmal AF (both P&lt;0.01). During the median follow-up period of 6.0 years, 255 patients (10%) were hospitalized for HF (annual incidence: 1.9% per person-year). Kaplan-Meier curves revealed that LVEF at enrollment could stratify the incidence of HF hospitalization in AF patients without pre-existing HF ( Figure ). Multivariable Cox regression analysis demonstrated that lower LVEF strata were independently associated with the risk of HF after adjustment by age, sex, AF type and CHA 2 DS 2 -VASc score ( Figure ). Conclusion: LVEF at enrollment could stratify the incidence of HF hospitalization in patients with AF, suggesting the importance of measuring LVEF in all AF patients even without HF.
Abstract 10985: Cardiopulmonary Assessment of COVID-19 Survivors Stratified by Acute Disease Severity and Post-Acute Symptoms
Authors: Yishay Szekely, Ehab Ghantous, Sapir SAdon, Vincenzo De Marzo, Piero Clavario, Italo Porto, Ernesto Crisafulli, Luca Vannini, Alejandro Quijada-Fumero, Ana Laynez-Carnicero, Mark Philip Cassar, Stefan Neubauer, Betty Raman, Shmuel Banai, Yan Topilsky
Journal: Circulation (2022)

Introduction: Studies have demonstrated a reduction in peak oxygen consumption (VO 2 ) post-acute COVID-19. We sought to determine the association between acute COVID-19 severity, post-acute symptoms and peak VO 2 after recovery. Methods: This study analyzed data from patients who recovered from COVID-19 and underwent cardiopulmonary exercise testing (CPET) as part of prospective studies in 5 centers across UK and Europe. Patients were asked to report current symptoms. Peak VO 2 , lung volumes, gas exchange, ventilatory efficiency, heart rate and O 2 pulse were measured in a standard symptom-limited incremental cycle ergometer CPET. Results: Among 417 patients examined 136±63 days after recovery from COVID-19, 164 (39%) were female. Mean age was 56.9±13.3 years. The spectrum of acute COVID-19 severity included critical (N=47; 11.3%), severe (180; 43.2%), moderate (75; 18.0%) and mild (115; 27.5%) illness. The most common post-acute symptom was dyspnea (200; 48%), followed by muscle pain (173; 41%). Mean peak respiratory exchange ratio was 1.13±0.1, and did not vary across acute disease severity or post-acute symptom status. There was no significant difference in peak VO 2 as % from predicted in mild to critical acute disease: 84.0±2.1%, 91.4±2.6%, 82.9±1.7% and 83.7±3.2%, respectively (p=0.06). Patients with dyspnea or muscle pain had each lower peak VO 2 as % from predicted, compared to patients free of the specific symptom (81.3±21.2% vs. 88.1±22.9%, p=0.002 and 78.6±19.1% vs. 88.2±22.0%, p&lt;0.0001, respectively). Both dyspnea and muscle pain were each associated with significantly lower peak heart rate and peak O 2 pulse, as well as lower lung volumes and gas exchange capacity, compared to patients free of the specific symptom. Conclusions: Common post-acute COVID-19 symptoms, and not acute disease severity, are associated with lower peak VO2 in CPET. Parameters related to both cardiac and pulmonary function seem to play a role in this reduced physical performance.
Abstract 11832: Impact of Social Determinants of Health on Sacubitril-Valsartan Accessibility: A Single Center Retrospective Review
Authors: Mian Tanveer Ud Din, Arinze Bosah, Richard Amoateng, nikos pappan, Grigorios Kalaitzidis, Craig Alpert, Divya Venkat
Journal: Circulation (2022)

Introduction: Angiotensin receptor-neprilysin inhibitors (ARNI) like sacubitril-valsartan reduce morbidity and mortality in heart failure with reduced ejection fraction (HFrEF). However, its higher cost relative to other guideline-directed medical therapy(GDMT) may ultimately limit access for patients, especially those impacted by social determinants of health (SDOH). Methods: A retrospective review of all patients at Allegheny General Hospital with HFrEF taking sacubitril-valsartan between January 1, 2016 and January 1, 2022 was conducted. Logistic regression was used to study the impact of various SDOH (Income, Employment, Transportation, Insurance) on medication adherence. Results: Out of 201 patients who met the inclusion criteria, ARNI was discontinued in 47 (23.4%) patients. Common reasons for discontinuation included cost/insurance coverage in 10 patients (21.3%), hypotension in 18 patients (38.3%), hyperkalemia (4.3%) and worsening renal function (10.6%). Lower income (OR:0.75 [95% CI 0.58 to 0.98] p = 0.03) and greater distance from clinic (OR:1.25 [95% CI 1.03 to 1.51] p = 0.02) was associated with higher odds of discontinuing ARNI. Discontinuation was observed more frequently in retired patients (55.3% vs 43.5%) compared to those actively employed. Compared to those with Medicare, Medicaid or no insurance, ARNI was continued more frequently in patients with private insurance. Conclusions: Patients with HFrEF live longer when treated with appropriate GDMT. However, given costs associated with novel agents, clinicians must acknowledge that SDOH substantially impact a patient’s ability to fill such expensive prescriptions regularly.
Abstract 12703: Transthyretin Amyloid Cardiomyopathy Among Patients Hospitalized for Heart Failure and Performance of an Adapted Wild-Type ATTR-CM Machine Learning Model: Findings From GWTG-HF
Authors: Anthony Peters, Nicole Solomon, Karen Chiswell, Gregg C Fonarow, Michel G Khouri, Alex Schepart, Lori Baylor, Jose Alvir, Marianna Bruno, Ahsan Huda, Larry A Allen, Kavita Sharma, Adam D Devore, Stephen J Greene
Journal: Circulation (2022)

Introduction: An 11-factor random forest model has been previously developed among ambulatory patients for identifying potential wild-type amyloidogenic TTR cardiomyopathy (wtATTR-CM), but model performance in a large sample of patients hospitalized for HF has not been evaluated. Methods: This study included Medicare beneficiaries hospitalized for HF in the Get With The Guidelines-HF® Registry from 2008-2019. Patients with and without a diagnosis of wtATTR-CM were compared. ICD9/10 codes were used as proxies to identify wtATTR-CM patients on inpatient/outpatient claims within ±6 months of hospitalization. Within a cohort matched 1:1 by age and sex, univariable logistic regression was used to evaluate relationships between each of the 11 factors of the established ATTR-CM model and wtATTR-CM. Discrimination and calibration of the 11-factor model was assessed. Results: Among 205,545 patients hospitalized for HF across 608 US hospitals, 627 patients (0.31%) had a diagnosis code for wtATTR-CM. wtATTR-CM patients were more likely to be male (69% vs 47%), Black race (23% vs 9%), and had lower systolic blood pressure (median 124 vs 139 mmHg). Univariable analysis within the 1:1 matched cohort of each of the 11-factors in the wtATTR-CM model found pericardial effusion, carpal tunnel syndrome, lumbar spinal stenosis, and elevated serum enzymes (e.g., troponin elevation) to be strongly associated with wtATTR-CM ( Figure, Panel A ). The 11-factor model showed modest discrimination with c-statistic of 0.65 ( Figure, Panel B ), with good calibration within the matched cohort ( Figure, Panel C ). Conclusions: Among patients hospitalized for HF in US practice, the number of wtATTR-CM as defined by diagnosis codes was low. Patients with a diagnosis of wtATTR-CM have a distinct clinical profile, and most factors within the prior 11-factor model were associated with greater odds of wtATTR-CM diagnosis. In this population, the wtATTR-CM model demonstrated modest discrimination.